Oral manifestations of nine individuals with Williams syndrome. A case series

Aims Williams syndrome (WS) is a congenital developmental disorder characterized, mainly, by distinctive facial features, cardiovascular anomalies, growth delay and a typical neurobehavioral profile. The oral manifestations have not been sufficiently described and, therefore, the aim of the current...

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Bibliographic Details
Published in:Special care in dentistry 2024-03, Vol.44 (2), p.438-449
Main Authors: Vavetsi, Konstantina, Panagopoulou, Olga, Koromantzos, Panagiotis, Fryssira, Ηelen, Bobetsis, Spyridon A., Emmanouil, Dimitris, Bobetsis, Yiorgos A.
Format: Article
Language:English
Subjects:
Adult
Case reports
case series
Congenital defects
Dental Caries
Dental occlusion
Dental plaque
Female
Gingivitis
Humans
Malocclusion
oral manifestations
Periodontal diseases
Phenotypes
Radiography
Tooth Abnormalities
Williams syndrome
Williams Syndrome - complications
Williams Syndrome - genetics
Young Adult
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Summary:Aims Williams syndrome (WS) is a congenital developmental disorder characterized, mainly, by distinctive facial features, cardiovascular anomalies, growth delay and a typical neurobehavioral profile. The oral manifestations have not been sufficiently described and, therefore, the aim of the current study was to present the clinical, radiographic and microbiological findings of individuals with WS. Methods and results A series of nine WS individuals (seven females) with mean age 21 years‐old were evaluated. A complete intraoral clinical examination, a radiographic analysis using panoramic and cephalometric x‐ray and a supra‐ and sub‐gingival microbiological profiling were performed. We observed abnormal tooth morphology, excessive interdental spacing, congenitally missing permanent teeth and malocclusion. High levels of DMFT were observed and gingivitis was present in all subjects. Bacteria related to periodontal disease were detected in dental plaque. Three patients were classified with a gingival phenotype type I according to the Maynard and Wilson classification. The sella turcica bridging was a novel finding for this group of patients. Conclusion Due to the elevated prevalence of gingivitis, caries and malocclusion, a multidisciplinary approach including dental follow‐ups should be the standard of care in WS patients.
ISSN:0275-1879
1754-4505
DOI:10.1111/scd.12881